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1.
J Am Acad Dermatol ; 8(1): 50-62, 1983 Jan.
Article in English | MEDLINE | ID: mdl-6826808

ABSTRACT

Eight cases of autopsy-proved malignant histiocytosis (MH) are reported, in which cutaneous involvement was a prominent finding at initial clinical presentation. In two patients, the disease remained confined to the skin for significant periods before systemic dissemination appeared. Immunohistochemical analysis of cutaneous infiltrates of MH showed uniform negativity of the atypical cells for lysozyme, immunoglobulin light chain, glycogen, and chloroacetate esterase content. In five cases, cytochemical stains for acid phosphatase, butyrate (nonspecific) esterase, chloroacetate esterase, and peroxidase activity were performed on bone marrow aspirates containing malignant cells; all demonstrated diffuse positivity for acid phosphatase and nonspecific esterase, and negativity for peroxidase and chloroacetate esterase. Touch-imprint preparations of cutaneous lesions in one of these cases yielded identical results. These findings indicate that cytochemical methods are preferable to immunohistochemical technics in identification of histiocytic malignancies and that foci of skin involvement may provide a useful source for such diagnostic evaluation.


Subject(s)
Lymphatic Diseases/pathology , Skin Neoplasms/pathology , Skin/pathology , Acid Phosphatase/analysis , Adult , Aged , Bone Marrow/pathology , Esterases/analysis , Female , Histocytochemistry , Humans , Liver/pathology , Lymphatic Diseases/metabolism , Lymphoid Tissue/pathology , Male , Middle Aged , Skin Neoplasms/metabolism
2.
J Am Acad Dermatol ; 7(4): 468-77, 1982 Oct.
Article in English | MEDLINE | ID: mdl-6754771

ABSTRACT

Tilorone hydrochloride was used to treat eleven patients with T cell cutaneous disease ranging from pre-Sézary syndrome to tumor-stage mycosis fungoides. Cutaneous histologic study, Sézary counts, delayed skin tests, patch tests, and quantitative T cell counts were monitored. The study revealed that the response to tilorone depends in part on the type and stage of disease, as well as the characteristics and responsiveness of the individual lymphocyte population. Patients with pre-Sézary syndrome are most likely to benefit from tilorone. The effect of tilorone on the T cell population is manifested by changes in responses to patch and skin tests, as well as histologic improvement. Intact immune responses and elevated levels of IgE may be important prognostic clinical parameters in these patients. Tilorone is ineffective in patients with mycosis fungoides and advanced Sézary syndrome. Keratopathy can be a limiting but reversible complication of therapy. The drug may provide an effective therapeutic step in the treatment of early T cell cutaneous disease or an adjunctive therapy to leukapheresis and chemotherapy.


Subject(s)
Fluorenes/therapeutic use , Lymphoproliferative Disorders/drug therapy , Skin Diseases/drug therapy , Tilorone/therapeutic use , Adult , Aged , Candida albicans/immunology , Female , Humans , Male , Middle Aged , Mycosis Fungoides/drug therapy , Mycosis Fungoides/pathology , Parapsoriasis/drug therapy , Parapsoriasis/pathology , Sezary Syndrome/drug therapy , Sezary Syndrome/pathology , Skin/pathology , Skin Diseases/pathology , Skin Neoplasms/drug therapy , Skin Neoplasms/pathology , Skin Tests , T-Lymphocytes/analysis , Trichophyton/immunology
3.
J Cutan Pathol ; 9(5): 293-302, 1982 Oct.
Article in English | MEDLINE | ID: mdl-6292273

ABSTRACT

Clinical and histopathologic data in 8 cases of epidermoid hidradenoma are presented. Most of the tumors were in the head and neck, and most of the patients were middle-aged and older adults. Generally, the lesions were asymptomatic nodules that sometimes showed ulceration or rapid growth. Despite having some histologic atypia or focal mitotic activity, the tumors were found to be benign on long-term evaluation. This epidermoid variant should not be otherwise differentiated from the benign group of solid-cystic hydradenomas, because cytologic variability did not predict a significant change in prognosis.


Subject(s)
Adenoma, Sweat Gland/pathology , Skin Neoplasms/pathology , Adenoma, Sweat Gland/diagnosis , Adenoma, Sweat Gland/surgery , Adolescent , Adult , Aged , Diagnosis, Differential , Female , Follow-Up Studies , Humans , Male , Middle Aged , Prognosis , Skin Neoplasms/diagnosis , Skin Neoplasms/surgery
4.
J Am Acad Dermatol ; 5(5): 571-81, 1981 Nov.
Article in English | MEDLINE | ID: mdl-6117589

ABSTRACT

Allergic granulomatosis is a distinct clinical syndrome occurring in adults with asthma, eosinophilia, and multisystem vasculitis. Atopy and drug sensitivity are other important features. The skin reactions are most commonly nodular and inflammatory lesions. A unique feature is the deep papulonodules, which may occur singly on the scalp or symmetrically on the extremities. Cutaneous findings may range from purpura to urticaria and ulceration. The most common histologic finding is the extravascular granuloma; however, necrotizing vasculitis of small vessels is seen, as well as periarteritis nodosa involving larger vessels of the skin. This varied histologic and clinical spectrum seen in patients with systemic allergic granulomatosis is a reflection of a unique host response to multiple antigens. The cutaneous findings imply that allergic granulomatosis probably represents a unique host response to the same causative and pathogenetic factors as are usually found in periarteritis nodosa and necrotizing vasculitis.


Subject(s)
Vasculitis, Leukocytoclastic, Cutaneous/pathology , Adult , Asthma/complications , Diagnosis, Differential , Eosinophilia/complications , Female , Granuloma/pathology , Humans , Male , Middle Aged , Polyarteritis Nodosa/pathology , Skin/pathology , Vasculitis, Leukocytoclastic, Cutaneous/complications
5.
Arch Dermatol ; 117(9): 540-2, 1981 Sep.
Article in English | MEDLINE | ID: mdl-7027965

ABSTRACT

Familial benign chronic pemphigus (Hailey and Hailey disease) is a rare, recalcitrant, often disabling genodermatosis that may not respond to conservative dermatologic therapy. We describe herein five patients with intertriginous familial benign chronic pemphigus who underwent excision and split-thickness skin grafting. All were men whose duration of disease ranged from one to 38 years. Follow-up evaluations ranging from ten months to nine years revealed no recurrence in graft sites in three patients, mild recurrence in one patient after eight years, and one death from pulmonary embolus in the postoperative period. The occurrence of familial benign chronic pemphigus around graft edges was a universal, but relatively minor, problem. Surgical excision provided definite relief from an otherwise disabling disease in four of our patients and a satisfactory improvement in life-style.


Subject(s)
Pemphigus/surgery , Skin Transplantation , Adult , Chronic Disease , Follow-Up Studies , Humans , Male , Middle Aged , Pemphigus/genetics
6.
Arch Dermatol ; 117(6): 338-40, 1981 Jun.
Article in English | MEDLINE | ID: mdl-6972736

ABSTRACT

Four cases of factitious crusting of the lips in women are reported. Two of the women had hemorrhagic crusts, and two had keratotic yellow crusts. All four patients had personality disturbances. Biting, picking, or unconscious licking of the lips may be the underlying mechanism for trauma and crust formation. This entity should be distinguished from contact cheilitis, actinic cheilitis, infectious cheilitis glandularis, and cheilitis granulomatosa. Some cases of exfoliative cheilitis may also be factitious. The presence of bizarre hemorrhagic or keratotic crusts on the lips should alert the clinician to a possible factitious origin, and a psychiatric evaluation should be done.


Subject(s)
Cheilitis/psychology , Personality Disorders/complications , Adolescent , Adult , Cheilitis/pathology , Diagnosis, Differential , Female , Humans , Lip Diseases/etiology , Lip Diseases/pathology , Oral Hemorrhage/etiology , Oral Hemorrhage/pathology
7.
Arch Dermatol ; 117(3): 158-9, 1981 Mar.
Article in English | MEDLINE | ID: mdl-7212730

ABSTRACT

A 42-year-old woman had blue telangiectases of the fingertips associated with myxedema. In addition to this clinical finding, the patient had abnormal Doppler ultrasound study findings analogous to those seen in peripheral vascular occlusion. With thyroid replacement therapy, the telangiectases disappeared, and a dramatic improvement was noted in the results of the Doppler studies. We believe that the clinical picture seen in our patient was caused by the deposition of mucin around small blood vessels.


Subject(s)
Fingers/blood supply , Myxedema/complications , Telangiectasis/etiology , Adult , Female , Humans , Telangiectasis/diagnosis , Telangiectasis/drug therapy , Thyroxine/therapeutic use , Ultrasonography
8.
J Am Acad Dermatol ; 4(2): 181-94, 1981 Feb.
Article in English | MEDLINE | ID: mdl-7217389

ABSTRACT

We have seen five adult patients with a clinical picture of recurrent histiocytic, cytophagic panniculitis, cytopenia, abnormal liver function tests, and a terminal, febrile bleeding diathesis. Originally thought to have Weber-Christian disease, these patients, we believe, represent a unique syndrome: lobular, histiocytic, cytophagic panniculitis. Erythrophagocytosis and cytophagocytosis are readily observed, but the cells do not show malignant features. Histiocytosis can be found at times in the bone marrow, lymph nodes, liver, spleen, and serosal tissues, as well as in the skin and subcutaneous tissues. The terminal hemorrhage in these patients is characterized by features of pancytopenia, liver failure, and intravascular coagulation. This disease may be separated from malignant histiocytosis by the chronic course, the primary involvement of the adipose tissue, and the benign histiocytes in the infiltrate. It has some similarities to other regional histiocytoses such as sinus histiocytosis, intestinal histiocytosis, and splenic histiocytosis.


Subject(s)
Hemorrhagic Disorders/etiology , Liver Diseases/etiology , Pancytopenia/etiology , Panniculitis, Nodular Nonsuppurative/diagnosis , Adult , Aged , Female , Fever/etiology , Histiocytes/pathology , Humans , Lymph Nodes/pathology , Male , Middle Aged , Panniculitis, Nodular Nonsuppurative/pathology , Panniculitis, Nodular Nonsuppurative/physiopathology , Phagocytosis , Recurrence , Syndrome
9.
Arch Dermatol ; 116(11): 1252-6, 1980 Nov.
Article in English | MEDLINE | ID: mdl-7002046

ABSTRACT

Four patients with ulcerative lichen planus of the feet underwent total excision and split-thickness skin grafting of the affected areas. One patient underwent grafting in 1951, and remained free of recurrent ulcers for 26 years. Another, who had an 18-year follow-up after grafting, required an additional procedure. The possibility of squamous cell carcinoma in ulcerative lichen planus is considerable, lending further support to the concept that excision with grafting is the treatment of choice for these lesions.


Subject(s)
Lichen Planus/surgery , Skin Transplantation , Adult , Aged , Female , Follow-Up Studies , Foot/surgery , Humans , Male , Middle Aged , Skin Ulcer/surgery
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